Faculty Bibliography

A sound performance validity test is accurate for detecting invalid neuropsychological test performance and relatively insensitive to actual cognitive ability or impairment. This study explored the relationship of several cognitive abilities to several performance indices on the Victoria Symptom Validity Test (VSVT), including accuracy and response latency. This cross-sectional study examined data from a mixed clinical sample of 88 adults identified as having valid neurocognitive test profiles via independent validity measures, and who completed the VSVT along with objective measures of working memory, processing speed, and verbal memory during their clinical neuropsychological evaluation. Results of linear regression analyses indicated that cognitive test performance accounted for 5% to 14% of total variance for VSVT performance across indices. Working memory was the only cognitive ability to predict significant, albeit minimal, variance on the VSVT response accuracy indices. Results show that VSVT performance is minimally predicted by working memory, processing speed, or delayed verbal memory recall.

Failure to diagnose transient ischemic attack (TIA) or stroke in a timely fashion is associated with significant patient morbidity and mortality. In the outpatient or clinic setting, we suspect that patients with minor, transient, and atypical manifestations of cerebrovascular disease are most prone to missed or delayed diagnosis. We therefore detail common stroke chameleon symptoms as well as atypical stroke presentations, broadly review new developments in the study of diagnostic error in the outpatient setting, suggest practical clinical strategies for diagnostic error reduction, and emphasize the need for rapid consultation of stroke specialists when appropriate. We also address the role of psychiatric disease and vascular risk factors in the diagnostic evaluation and treatment of suspected stroke/TIA patients. We advocate incorporating diagnostic time-outs into clinical practice to assure that the diagnosis of TIA or stroke is considered in all relevant patient encounters after a detailed history and examination are conducted in the outpatient setting.

Background/Purpose: Sudden unexpected death in epilepsy (SUDEP) is a sudden death in epilepsy patients not explained by status epilepticus, trauma, or any another known cause. In Dravet syndrome (DS) the incidence of SUDEP is about 6- fold higher than in other forms of epilepsy. The objective of this study was to compare the incidence of SUDEP in FFA-treated DS patients with literature reports of SUDEP incidence in patients with DS receiving anticonvulsive treatment without FFA.
Method(s): For the study group without FFA, publications were identified in PubMed searching 'Dravet [title] AND (mortality OR death OR SUDEP).' The FFA-treated population comprised patients from 3 sources: international phase 3 clinical trials, US and European Early Access Programs (EAPs), and a long-term, open-label study spanning 32 years. The incidence of SUDEP was expressed as deaths per 1,000 person-years of observation.
Result(s): Nine studies describing the incidence of SUDEP in DS were identified. Cooper (Cooper MS, Epilepsy Res 2016;128:43-47) was considered the most rigorous, reporting a SUDEP rate of 9.32 per 1000 person-years (98% CI, 4.46- 19.45). 732 patients treated with fenfluramine provided 1185.3 person-years. The FFA-SUDEP rate was below the lower limit of 98% CI reported by Cooper, whereas the SUDEP rate before starting FFA was similar to the literature numbers.
Conclusion(s): Results show a lower incidence of SUDEP and all-cause mortality in the FFA-treated population compared to patients without FFA of the literature. Further research is warranted to clarify influencing factors on SUDEP to reduce its risks. The data were first presented at AES 2020 (Virtual 74th American Epilepsy Society Annual Meeting)

OBJECTIVE:To develop evidence-informed, expert consensus research diagnostic criteria for traumatic encephalopathy syndrome (TES), the clinical disorder associated with neuropathologically diagnosed chronic traumatic encephalopathy (CTE). METHODS:April, 2019. Before consensus, panelists reviewed evidence from all published cases of CTE with neuropathologic confirmation, and they examined the predictive validity data on clinical features in relation to CTE pathology from a large clinicopathologic study (n = 298). RESULTS:Consensus was achieved in 4 rounds of the Delphi procedure. Diagnosis of TES requires (1) substantial exposure to repetitive head impacts (RHIs) from contact sports, military service, or other causes; (2) core clinical features of cognitive impairment (in episodic memory and/or executive functioning) and/or neurobehavioral dysregulation; (3) a progressive course; and (4) that the clinical features are not fully accounted for by any other neurologic, psychiatric, or medical conditions. For those meeting criteria for TES, functional dependence is graded on 5 levels, ranging from independent to severe dementia. A provisional level of certainty for CTE pathology is determined based on specific RHI exposure thresholds, core clinical features, functional status, and additional supportive features, including delayed onset, motor signs, and psychiatric features. CONCLUSIONS:New consensus diagnostic criteria for TES were developed with a primary goal of facilitating future CTE research. These criteria will be revised as updated clinical and pathologic information and in vivo biomarkers become available.

BACKGROUND AND OBJECTIVES:We compared heart rate variability (HRV) in sudden unexpected death in epilepsy (SUDEP) cases and living epilepsy controls. METHODS:This international, multicenter, retrospective, nested case-control study examined patients admitted for video-EEG monitoring (VEM) between January 1, 2003, and December 31, 2014, and subsequently died of SUDEP. Time domain and frequency domain components were extracted from 5-minute interictal ECG recordings during sleep and wakefulness from SUDEP cases and controls. RESULTS:= 0.209). CONCLUSIONS:Reduced short-term LFP, which is a validated biomarker for sudden death, was associated with SUDEP. Increased HFP was associated with longer survival and may be cardioprotective in SUDEP. HRV quantification may help stratify individual SUDEP risk. CLASSIFICATION OF EVIDENCE:This study provides Class III evidence that in patients with epilepsy, some measures of HRV are associated with SUDEP.